Prevalence of long QT syndrome and other cardiac defects in deaf-mute children

Long QT syndrome is considered a fatal disease because of its association with ventricular arrhythmias and sudden cardiac death. Objectives of study were to determine the prevalence of long QT syndrome and other heart diseases, in deaf-mute children. A Cross-sectional descriptive study was conducted at Cholistan special education centre and Cardiology department, Sheikh Zayed hospital Rahim Yar Khan, Pakistan in September 2006. A total of 104 congenitally deaf-mute children were assessed. Height, weight and blood pressure measured, 12-lead electrocardiogram done and QTc calculated using Bazette's formula. Children with prolonged QTc underwent 24-hour ambulatory ECG recording. All were auscultated following complete protocol. A child with murmur was further evaluated with colour Doppler echocardiography. Audiometry was performed on all the children and the result interpreted according to WHO recommendations. Diagnosis of LQTS was based on Revised Schwartz criteria. Out of 104 children, 62 were male with mean age 11.89 yrs. The average systolic and diastolic BP was 97/67 mmHg. Average height was 126 Cm. All children had moderate to severe bilateral sensorineural hearing loss [40-80 dB]. One child had associated Patent Ductus Arteriosis. Fifteen had an innocent murmur. Prevalence of congenital heart disease was found to be 0.1/1000. Four children had QT interval more than 440 mSec, [range 0.46-0.47 mSec.]. Both genders were equally affected. Three children had high probability of LQTS and one had intermediate probability. Screening of family of these 4 patients showed prolonged QT interval in the sibling of one patient. Our study highlights the significant prevalence of Jervell Lange-Nielsen Syndrome in Pakistani deaf-mute children, which may be associated to the high level of consanguinity in this region. Awareness of this syndrome among health care providers is needed as timely diagnosis and subsequent treatment may prevent fatal complications.

Trends of utilizing medication, recommended by evidence based guidelines, for myocardial infarction, in a tertiary care hospital, "too far but not too dark"

Background: acute myocardial infarction [MI] is a major health problem with a substantial mortality and morbidity. Numerous guidelines have been established that should be followed in the management of acute MI

Objective: to determine whether the current practice in managing patients admitted with acute MI in a tertiary care hospital is evidence based compliant

Patients and Methods: this retrospective study was based on the record of the patients with diagnosis ofMI admittedbetween January to June 2007, at Sheikh Zayed Hospital, Rahim Yar Khan, who fulfilled the predefined criteria

Results: Total number of cases were 58, mean age of the study subjects was 47+ 8.65 years, with age range of 16 to 95 years. It was observed that 81% of study subjects were male. Half of the patients belonged to lower income group; Laborer and house wives were 3 1 % and 19% respectively. Sixteen percent of patients were hypertensive and diabetic. 19 % were smoker and 5% had family history of coronary artery disease. Anterior and inferior wall MI were reported in 64% and 32% of the patients, respectively. Injection Streptokinase [SK] was given to 52% of the patients. Aspirin, clopidogril and nitrates were given to all patients, whereas 34 % and 36% were prescribed Beta blocker [BB] and statins, respectively. Angiotensin converting enzyme inhibitor [ACEI] was given in 43% patients

Conclusion: SK, the only means of acute revascularization was given in only 52% patients mainly due to delayed presentation. Use ofAspirin is an unbeatable target and given to all patients. Use of BB and statins in < 40% and ACEI in<50% is not optimal. However, these trends of cardiac medication in a tertiary care hospital with limited provision, reflects the diffusion of light of evidence based medicine into darkness of periphery